Survival Profiles of Patients With Frontotemporal Dementia and Motor Neuron Disease

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Abstract
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) form a clinicopathologic continuum. Clinically, features of motor neuron disease (MND) such as ALS or primary lateral sclerosis (PLS) may develop in patients with a behavioral or language variant of FTD, and prominent behavioral and language deficits can emerge in the course of ALS.1-4 Pathologically, tau-negative and α-synuclein–negative cases of FTD have characteristic lesions immunoreactive to TAR DNA-binding protein of approximately 43 kDa (TDP-43), and TDP-43–immunoreactive (TDP-ir) inclusions are also the characteristic lesions in the spinal cord and motor cortex of patients with ALS.5-7 The overlapping clinical and pathologic features in frontotemporal lobar degeneration with ubiquitin/TDP-43–positive inclusions and ALS suggest that the 2 disorders represent a TDP-43 proteinopathy spectrum despite the differences in initial clinical presentation.8,9 Patients who develop FTD followed by symptoms of MND are commonly given the diagnosis of FTD-MND,10,11 and those who develop ALS before dementia are frequently diagnosed as having ALS with dementia (ALS-D).12